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Journal of Hematology & Oncology Jan 2018Solitary plasmacytoma is an infrequent form of plasma cell dyscrasia that presents as a single mass of monoclonal plasma cells, located either extramedullary or... (Review)
Review
Solitary plasmacytoma is an infrequent form of plasma cell dyscrasia that presents as a single mass of monoclonal plasma cells, located either extramedullary or intraosseous. In some patients, a bone marrow aspiration can detect a low monoclonal plasma cell infiltration which indicates a high risk of early progression to an overt myeloma disease. Before treatment initiation, whole body positron emission tomography-computed tomography or magnetic resonance imaging should be performed to exclude the presence of additional malignant lesions. For decades, treatment has been based on high-dose radiation, but studies exploring the potential benefit of systemic therapies for high-risk patients are urgently needed. In this review, a panel of expert European hematologists updates the recommendations on the diagnosis and management of patients with solitary plasmacytoma.
Topics: Disease Management; Europe; Humans; Magnetic Resonance Imaging; Plasmacytoma; Positron Emission Tomography Computed Tomography; Prognosis; Treatment Outcome
PubMed: 29338789
DOI: 10.1186/s13045-017-0549-1 -
British Journal of Haematology Aug 2021In this review, two types of soft-tissue involvement in multiple myeloma are defined: (i) extramedullary (EMD) with haematogenous spread involving only soft tissues and... (Review)
Review
In this review, two types of soft-tissue involvement in multiple myeloma are defined: (i) extramedullary (EMD) with haematogenous spread involving only soft tissues and (ii) paraskeletal (PS) with tumour masses arising from skeletal lesions. The incidence of EMD and PS plasmacytomas at diagnosis ranges from 1·7% to 4·5% and 7% to 34·4% respectively. EMD disease is often associated with high-risk cytogenetics, resistance to therapy and worse prognosis than in PS involvement. In patients with PS involvement a proteasome inhibitor-based regimen may be the best option followed by autologous stem cell transplantation (ASCT) in transplant eligible patients. In patients with EMD disease who are not eligible for ASCT, a proteasome inhibitor-based regimen such as lenalidomide-bortezomib-dexamethasone (RVD) may be the best option, while for those eligible for high-dose therapy a myeloma/lymphoma-like regimen such as bortezomib, thalidomide and dexamethasone (VTD)-RVD/cisplatin, doxorubicin, cyclophosphamide and etoposide (PACE) followed by SCT should be considered. In both EMD and PS disease at relapse many strategies have been tried, but this remains a high-unmet need population.
Topics: Animals; Antineoplastic Combined Chemotherapy Protocols; Bortezomib; Cisplatin; Cyclophosphamide; Dexamethasone; Disease Management; Doxorubicin; Etoposide; Hematopoietic Stem Cell Transplantation; Humans; Lenalidomide; Multiple Myeloma; Plasmacytoma; Prognosis; Transplantation, Autologous
PubMed: 33724461
DOI: 10.1111/bjh.17338 -
The American Journal of Surgical... Oct 2022Epstein-Barr virus (EBV)-positive plasmacytoma is a rare plasma cell neoplasm. It remains unclear whether EBV-positive plasmacytoma represents a distinct entity or a...
Epstein-Barr virus (EBV)-positive plasmacytoma is a rare plasma cell neoplasm. It remains unclear whether EBV-positive plasmacytoma represents a distinct entity or a variant of plasmacytoma. It shares morphologic features with plasmablastic lymphoma (PBL) and may cause diagnostic uncertainty. To better understand EBV-positive plasmacytoma and explore diagnostic criteria, this study describes 19 cases of EBV-positive plasmacytoma, compared with 27 cases of EBV-negative plasmacytoma and 48 cases of EBV-positive PBL. We reviewed the clinicopathologic findings and performed immunohistochemistry, in situ hybridization for EBV, fluorescence in situ hybridization for MYC , and next-generation sequencing. We found that 63.2% of patients with EBV-positive plasmacytoma were immunocompromised. Anaplastic features were observed in 7/19 cases. MYC rearrangement was found in 25.0% of them, and extra copies of MYC in 81.3%. EBV-positive and EBV-negative plasmacytomas possessed similar clinicopathologic features, except more frequent cytologic atypia, bone involvement and MYC aberrations in the former group. The survival rate of patients with EBV-positive plasmacytoma was comparable to that of patients with EBV-negative plasmacytoma. In comparison to PBL, EBV-positive plasmacytoma is less commonly associated with a "starry-sky" appearance, necrosis, absence of light chain expression, and a high Ki67 index (>75%). The most recurrently mutated genes/signaling pathways in EBV-positive plasmacytoma are epigenetic regulators, MAPK pathway, and DNA damage response, while the most frequently reported mutations in PBL are not observed. Collectively, EBV-positive plasmacytoma should be regarded as a biological variant of plasmacytoma. Thorough morphologic examination remains the cornerstone for distinguishing EBV-positive plasmacytoma and PBL, and molecular studies can be a valuable complementary tool.
Topics: Epstein-Barr Virus Infections; Herpesvirus 4, Human; Humans; In Situ Hybridization, Fluorescence; Ki-67 Antigen; Plasmacytoma
PubMed: 35650679
DOI: 10.1097/PAS.0000000000001923 -
Urology Jul 1989The case of a sixty-five-year-old man with multiple myeloma and a testicular plasmacytoma is described. This represents the thirty fourth reported case of testicular... (Review)
Review
The case of a sixty-five-year-old man with multiple myeloma and a testicular plasmacytoma is described. This represents the thirty fourth reported case of testicular plasmacytoma and the first in which immunoperoxidase histochemistry has been used to demonstrate that the testicular plasma cells contain immunoglobulin of the same isotype as the patient's paraprotein. The clinical and morphologic features of previously reported testicular plasmacytoma are reviewed.
Topics: Aged; Female; Humans; Immunoenzyme Techniques; Immunoglobulins; Male; Plasmacytoma; Testicular Neoplasms
PubMed: 2665287
DOI: 10.1016/0090-4295(89)90159-3 -
The Neuroradiology Journal Sep 2014This pictorial review describes the spectrum of CT/MR imaging findings of solitary extramedullary and bone plasmacytomas in different locations related to... (Review)
Review
This pictorial review describes the spectrum of CT/MR imaging findings of solitary extramedullary and bone plasmacytomas in different locations related to neuroradiology. Plasmacytoma is considered a counterpart of multiple myeloma that is described as a solitary and discrete mass of monoclonal neoplastic plasma cells. It may arise from osseous (medullary) or extramedullary sites. Isolated extramedullary plasmacytoma is very rare and comprises less than 4% of all plasma cellular diseases of which more than 80% are localized to the submucosal lymphoid tissue of the nasopharynx, nasal cavity and paranasal sinuses. We will demonstrate imaging findings in ten histopathologically proven plasmacytomas in different locations related to neuroradiology. Extramedullary and osseous plasmacytoma show nonspecific CT and MR imaging findings. MR is the preferred modality for evaluation due to better soft tissue contrast. Features that may suggest the diagnosis of plasmacytoma are bulky soft tissue mass and relatively isointense signal on T2-weighted MR images due to high cellularity.
Topics: Bone Marrow Neoplasms; Humans; Magnetic Resonance Imaging; Nasopharyngeal Neoplasms; Neuroimaging; Paranasal Sinus Neoplasms; Plasmacytoma; Skull Neoplasms; Tomography, X-Ray Computed
PubMed: 25196616
DOI: 10.15274/NRJ-2014-10078 -
TheScientificWorldJournal 2012Solitary plasmacytoma (SP) is characterized by a mass of neoplastic monoclonal plasma cells in either bone (SBP) or soft tissue without evidence of systemic disease... (Review)
Review
Solitary plasmacytoma (SP) is characterized by a mass of neoplastic monoclonal plasma cells in either bone (SBP) or soft tissue without evidence of systemic disease attributing to myeloma. Biopsy confirmation of a monoclonal plasma cell infiltration from a single site is required for diagnosis. The common presentation of SBP is in the axial skeleton, whereas the extramedullary plasmacytoma (EMP) is usually seen in the head and neck. The ratio of SP seen at males to females is 2 : 1 and the median age of patients is 55 years. The incidence rate of SP in black race is approximately 30% higher than the white race. Incidence rate increases exponentially by advancing age. SBP has a significant higher risk for progression to myeloma, and the choice of treatment is radiotherapy (RT) that is applied with curative intent at min. 4000 cGy. By only RT application, long-term disease-free survival (DFS) is possible for approximately 30% of patients with SBP and 65% of patients with EMP.
Topics: Bone Neoplasms; Female; Humans; Male; Middle Aged; Plasmacytoma
PubMed: 22654647
DOI: 10.1100/2012/895765 -
BMC Cancer Apr 2020To study the histological structure and immunohistochemical (IHC) parameters of the plasmacytoma tumour substrate in patients with multiple myeloma (MM).
BACKGROUND
To study the histological structure and immunohistochemical (IHC) parameters of the plasmacytoma tumour substrate in patients with multiple myeloma (MM).
METHODS
The study included 21 patients (10 men/11 women) aged 23 to 73 years old with newly diagnosed MM complicated by plasmacytoma. Bone plasmacytoma was diagnosed in 14 patients, and extramedullary plasmacytoma was diagnosed in 7 patients. Plasmacytoma tissue specimens were examined using a LEICA DM4000B microscope. Anti-CD56, anti-CD166, anti-CXCR4, anti-Ki-67, and anti-c-MYC antibodies were used for IHC study of plasmacytoma biopsies.
RESULTS
When comparing the morphology of bone and extramedullary plasmacytoma, no significant differences were revealed; however, the substrate of extramedullary plasmacytoma was more often represented by tumour cells with an immature morphology than was the bone plasmacytoma substrate (57.1% vs. 28.6%, respectively). We revealed a significant difference in the expression of CD166 between bone and extramedullary plasmacytoma. The mean values of CD166 expression in bone plasmacytoma cells were significantly higher (36.29 ± 7.61% versus 9.57 ± 8.46%, respectively; p = 0.033) than those in extramedullary plasmacytoma cells. We noticed that in extramedullary plasmacytoma cells, there were higher values for the Ki-67 index than in bone plasmacytoma cells, and this result was independent of cell morphology.
CONCLUSION
The mechanisms involved in the dissemination of tumour plasma cells are currently unexplored. Even in such a small sample, some differences in expression could be identified, which may indicate that different mechanisms lead to the formation of bone and extramedullary plasmacytomas. Specifically, the expression of CD166 in extramedullary plasmacytoma cells was almost 4 times lower than that in bone plasmacytoma cells, which may indicate the involvement of CD166 in the mechanisms of bone destruction. The proliferative activity of extramedullary plasmacytoma cells was shown to be higher than that of bone plasmacytoma cells.
Topics: Adult; Aged; Female; Follow-Up Studies; Humans; Immunohistochemistry; Male; Middle Aged; Multiple Myeloma; Plasmacytoma; Prognosis; Young Adult
PubMed: 32321465
DOI: 10.1186/s12885-020-06870-w -
Ear, Nose, & Throat Journal May 2021The aim is to analyze the clinical outcomes of patients with extramedullary plasmacytoma (EMP) and review the characteristics of this disease. We retrospectively... (Review)
Review
The aim is to analyze the clinical outcomes of patients with extramedullary plasmacytoma (EMP) and review the characteristics of this disease. We retrospectively reviewed the clinical data of a cohort of 22 patients diagnosed with EMP between 1983 and 2017. Baseline characteristics and progression data were collected to calculate the incidence of progression to multiple myeloma (MM) and to analyze survival rates and outcomes. Previous major cohort studies were reviewed and compared with this study. Extramedullary plasmacytomas were located in the head and neck in 17 (77%) of the 22 patients. The median time between onset and diagnosis was 7 months, and the median age at diagnosis was 52.5 years (range 15-72 years). Extramedullary plasmacytoma occurred more frequently in men with an approximate ratio of 2.1:1. The majority of patients underwent surgery (13/22, 59.1%) and half received radiation (11/22, 50%). A small proportion of patients received chemotherapy (2/22, 9.1%). There was a median follow-up time of 98.5 months. The 5-year overall survival, progression-free survival, and MM-free survival rates were 84.2%, 67.3% and 75.9%, respectively. Complete resection without major functional damage is a promising option that can favorably improve prognosis in patients with resectable disease. Patients with regional lymph node metastasis or positive immunoelectrophoresis results require more aggressive treatment and may have poorer prognosis.
Topics: Adolescent; Adult; Aged; China; Female; Head and Neck Neoplasms; Humans; Incidence; Male; Middle Aged; Plasmacytoma; Prognosis; Retrospective Studies; Survival Rate; Young Adult
PubMed: 32941076
DOI: 10.1177/0145561320950587 -
Head and Neck Pathology Mar 2016Hematolymphoid neoplasms of the sinonasal tract are rare and the majority represents non-Hodgkin lymphomas. This review will focus on morphologic, immunophenotypic, and... (Review)
Review
Hematolymphoid neoplasms of the sinonasal tract are rare and the majority represents non-Hodgkin lymphomas. This review will focus on morphologic, immunophenotypic, and genetic characteristics of the most common types of non-Hodgkin lymphoma, namely diffuse large B cell lymphoma and extranodal natural killer/T-cell lymphoma, nasal type, but also include the discussion of less frequent other hematolymphoid entities, such as extranodal plasmacytomas and Rosai-Dorfman disease.
Topics: Histiocytosis, Sinus; Humans; Lymphoma, Non-Hodgkin; Nose Diseases; Paranasal Sinus Neoplasms; Plasmacytoma
PubMed: 26830397
DOI: 10.1007/s12105-016-0698-5 -
British Journal of Haematology Jul 2020
Topics: Aged; Female; Humans; Multiple Myeloma; Plasmacytoma
PubMed: 32301114
DOI: 10.1111/bjh.16646